Abstract
Introduction Ischemic choroidal diseases are an underdiagnosed entity. The clinical pattern varies
according to the size and the localisation of the affected vascular structure.
Clinical Presentation In eyes with occlusion of the long posterior ciliary arteries, characteristic triangular
patches of choroidal ischemia (Amalric sign) are seen, which in the course of time
merge into well-defined areas of atrophy of the retinal pigment epithelium. Above
the non-perfused choroidal areas, hyperpigmented, grouped lines appear (Siegrist streaks).
Circumscribed ischemia of smaller choroidal arterioles and capillary vessels appears
as multifocal, yellowish lesions in the posterior fundus (Elschnig spots). Vortex
vein occlusion becomes manifest as exudative haemorrhagic choroidal swelling in the
periphery.
Causes of Choroidal Ischemia Apart from arterial hypertension as a major risk factor, some immunological disorders
such as giant cell arteritis and systemic lupus erythematosus and haematological pathologies
also affect choroidal perfusion. Furthermore, choroidal ischemia occurs due to local
inflammation, as found in eyes with acute multifocal posterior placoid pigment epitheliopathy
(APMPPE). Rarely, choroidal infarction is of iatrogenic origin or drug-induced. Recent
advances in imaging, such as the introduction of enhanced depth imaging optical coherence
tomography (EDI-OCT) and OCT angiography (OCT-A), have improved the visualisation
of the choroidal vasculature and complement the classical angiographic procedures.
In patients with age-related macular degeneration (AMD) and diabetes, some changes
in choroidal blood flow and vascular structure have also been noted. While in AMD
the choroidal pathologies correlate with the disease progression and the functional
prognosis, the pathophysiological relationship between diabetic choroidopathy
and retinopathy is currently unclear.
Management and Conclusion With regard to the limited therapeutic options for choroidal ischemia, optimisation
of the cardiovascular risk profile and the management of accompanying ocular and systemic
diseases are essential.
Key words
choroid - angiography - choroidal infarction - vascular occlusion - ischemia
